title: A taste of periodic fever syndromes.
abstract: Periodic fevers are acquired or inherited disorders of innate immunity, which were first described in the 1940s. The patients are typically young at onset and have regularly recurring fevers for a few days to a few weeks with systemic inflammatory symptoms that are interrupted by symptom-free periods. There is a variety of clinical manifestations including gastrointestinal complaints, myalgias, arthralgias, and rash. A differential diagnosis in these patients may include recurrent infections, other inflammatory disorders, and neoplastic disease. This clinical review focuses on a sample of autoinflammatory disorders including familial Mediterranean fever, tumor necrosis factor receptor 1-associated periodic syndrome, hyperimmunoglobulinemia D syndrome, the cryopyrin-associated periodic syndrome, and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. We review the basics, pertinent clinical and laboratory features, and management of each entity.
authors.affiliation: Department of Emergency Medicine, University of Illinois College of Medicine at Peoria, OSF Saint Francis Medical Center, Peoria, IL 61637, USA. akoyfman8@gmail.com
authors.forename: Alex
authors.initials: A
authors.lastname: Koyfman
authors.forename: Emily
authors.initials: E
authors.lastname: Lovallo
authors.forename: Melissa M
authors.initials: MM
authors.lastname: Hazen
authors.forename: Vincent W
authors.initials: VW
authors.lastname: Chiang
mesh_terms: D000686:Amyloidosis / Q000209:etiology; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D002352:Carrier Proteins / Q000235:genetics; D003078:Colchicine / Q000627:therapeutic use; D003080:Cold Temperature / Q000009:adverse effects; D056587:Cryopyrin-Associated Periodic Syndromes / Q000097:blood / Q000175:diagnosis / Q000235:genetics; D004630:Emergencies; D000068800:Etanercept; D010505:Familial Mediterranean Fever / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D005334:Fever / Q000145:classification / Q000175:diagnosis* / Q000235:genetics; D056660:Hereditary Autoinflammatory Diseases / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use; D008199:Lymphadenitis / Q000209:etiology; D054078:Mevalonate Kinase Deficiency / Q000097:blood / Q000175:diagnosis / Q000235:genetics; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D010507:Periodicity; D010612:Pharyngitis / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000235:genetics; D013281:Stomatitis, Aphthous / Q000209:etiology
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D002352:Carrier Proteins; D007074:Immunoglobulin G; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human; D018124:Receptors, Tumor Necrosis Factor; D047888:Receptors, Tumor Necrosis Factor, Type I; D000068800:Etanercept; D003078:Colchicine; D011241:Prednisone